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Stable long-term risk of leukaemia in patients with severe congenital neutropenia maintained on G-CSF therapy

  1. Philip S. Rosenberg1,†,
  2. Cornelia Zeidler2,†,
  3. Audrey A. Bolyard3,
  4. Blanche P. Alter4,
  5. Mary A. Bonilla5,
  6. Laurence A. Boxer6,
  7. Yigal Dror7,
  8. Sally Kinsey8,
  9. Daniel C. Link9,
  10. Peter E. Newburger10,
  11. Akiko Shimamura11,
  12. Karl Welte2,†,
  13. David C. Dale3,†

Article first published online: 29 APR 2010

DOI: 10.1111/j.1365-2141.2010.08216.x

Issue

British Journal of Haematology

British Journal of Haematology

Volume 150, Issue 2, pages 196–199, July 2010

Additional Information

How to Cite

Rosenberg, P. S., Zeidler, C., Bolyard, A. A., Alter, B. P., Bonilla, M. A., Boxer, L. A., Dror, Y., Kinsey, S., Link, D. C., Newburger, P. E., Shimamura, A., Welte, K. and Dale, D. C. (2010), Stable long-term risk of leukaemia in patients with severe congenital neutropenia maintained on G-CSF therapy. British Journal of Haematology, 150: 196–199. doi: 10.1111/j.1365-2141.2010.08216.x

Author Information

  1. 1

    Biostatistics Branch, National Cancer Institute, Rockville, MD, USA

  2. 2

    Kinderklinik, Medizinische Hochschule, Carl-Neuberg-Str. 1, Hannover, Germany

  3. 3

    Department of Medicine, University of Washington, Seattle, WA

  4. 4

    Clinical Genetics Branch, National Cancer Institute, Rockville, MD

  5. 5

    St. Joseph’s Children’s Hospital, Pediatric Hematology Oncology, Paterson NJ

  6. 6

    University of Michigan, Pediatric Hematology/Oncology, Ann Arbor, MI, USA

  7. 7

    Hospital for Sick Children, The University of Toronto, Toronto, ON, Canada

  8. 8

    Paediatric Haematology, St. James’s University Hospital, Leeds, UK

  9. 9

    Washington University School of Medicine, Division of Bone Marrow Transplantation, St. Louis, MO

  10. 10

    University of Massachusetts Medical School, Worcester, MA

  11. 11

    Fred Hutchinson Cancer Research Center, Seattle, WA, USA

  1. †

    These authors contributed equally.

*Philip S. Rosenberg, Biostatistics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, 6120 Executive Blvd, EPS 8020, Rockville MD 20852, USA. E-mail: rosenbep@mail.nih.gov

Publication History

  1. Issue published online: 1 JUL 2010
  2. Article first published online: 29 APR 2010
  3. Received 1 February 2010; accepted for publication 17 March 2010

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Keywords:

  • severe congenital neutropenia;
  • acute myeloid leukaemia;
  • myelodysplastic syndromes;
  • granulocyte colony-stimulating factor

Summary

In severe congenital neutropenia (SCN), long-term therapy with granulocyte colony-stimulating factor (G-CSF) has reduced mortality from sepsis, revealing an underlying predisposition to myelodysplastic syndrome and acute myeloid leukaemia (MDS/AML). We have reported the early pattern of evolution to MDS/AML, but the long-term risk remains uncertain. We updated a prospective study of 374 SCN patients on long-term G-CSF enrolled in the Severe Chronic Neutropenia International Registry. Long-term, the annual risk of MDS/AML attained a plateau (2·3%/year after 10 years). This risk now appears similar to, rather than higher than, the risk of AML in Fanconi anaemia and dyskeratosis congenita.

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